Mitochondrial protein biogenesis in the synapse is supported by local translation

• DOI: 10.15252/embr.201948882
• Publication Date: 2020-06-19T07:35:40Z
• Authors: Bozena Kuzniewska; Dominik Cysewski; Michal Wasilewski; Paulina Sakowska; Jacek Milek; Tomasz M Kulinski; Maciej Winiarski; Pawel Kozielewicz; Ewelina Knapska; Michal Dadlez; Agnieszka Chacinska; Andrzej Dziembowski; Magdalena Dziembo...
• Keywords: Mice, Knockout; Mitochondrial Proteins; Proteomics; 0301 basic medicine; Fragile X Mental Retardation Protein; Mice; 03 medical and health sciences; Fragile X Syndrome; Synapses; Animals; Reports

Synapses are the regions of neuron that enable transmission and propagation action potentials on cost high energy consumption elevated demand for mitochondrial ATP production. The rapid changes in local energetic requirements at dendritic spines imply role mitochondria maintenance their homeostasis. Using global proteomic analysis supported with complementary experimental approaches, we show an essential pool proteins is locally produced synapse indicating protein biogenesis takes place to maintain functional axons dendrites. Furthermore, stimulation synaptoneurosomes induces synthesis transported incorporated into supercomplexes respiratory chain. Importantly, a mouse model fragile X syndrome, Fmr1 KO mice, common disease associated dysregulation synaptic synthesis, observed altered morphology respiration rates mitochondria. That indicates production plays functions.